Solitary fibrous tumor of the pleura with a minute malignant component and diaphragmatic vascular supply: Report of a case

We resected a fist-sized, solitary fibrous tumor of the pleura (SFTP) with a minute malignant component, following percutaneous embolization of its feeding artery. The tumor had macroscopic characteristics of a benign SFTP, and most parts of it were microscopically benign. However, further careful pathological examination revealed a minute malignant component in its periphery. We report this case to show that large and mostly benign SFTPs may contain malignant components, which can be overlooked. Thus, large SFTPs should be resected in consideration of this possibility.     

Successful diagnosis of diaphragmatic solitary fibrous tumor of the pleura by preoperative ultrasonography

The diaphragm is an uncommon site for solitary fibrous tumors of the pleura (SFTPs). Diaphragmatic SFTPs are usually found as pedunculated tumors with a clear margin with their surrounding structures. However, preoperative diagnosis of the tumor is difficult because their morphological features are not well detected on radiological images. We presented a case of diaphragmatic SFTP that was successfully diagnosed by ultrasonography (US). The US revealed a well-circumscribed mass with the feeding arteries leading from the diaphragm. Subsequently, this finding was confirmed histopathologically after resection. US can be less invasive and more sensitive than radiological examinations for diagnosing diaphragmatic SFTPs. To our knowledge, this is the first report of a preoperative diagnosis of diaphragmatic SFTP by US.     

Contralateral recurrence of a malignant solitary fibrous tumor of the pleura

We present an unusual case of a contralateral recurrence of malignant solitary fibrous tumor of the pleura (SFTP) nine years after a complete resection. Recurrence of malignant SFTP has already been described, but is usually localized. In our case the patient underwent surgical resection for a malignant SFTP of the left upper lobe in 2000. Nine years later computed tomography (CT)-scans showed lesions that were suspicious of tumor recurrence in the right lung. Thoracoscopy, wedge-resections and pathological findings revealed four nodules of a malignant SFTP of the right middle and lower lobe, histopathologically identical to the tumor, which had been resected nine years ago. A coincidental mucinous bronchioloalveolar carcinoma of the left lower lobe was resected by thoracotomy. To our knowledge this is the first report of contralateral recurrence of a malignant SFTP years after complete resection in the literature. The possibility of a new primary tumor on the right with local metastasis could not be excluded in the clinical and histopathological examinations. Therefore, contralateral recurrence of malignant SFTP should be considered in the postoperative follow-up even years after complete resection.     

电视胸腔镜手术治疗胸膜孤立性纤维瘤的临床分析

目的探讨电视胸腔镜手术（VATS）治疗胸膜孤立性纤维瘤（SFTP）的价值,总结微刨治疗经验。方法1999年2月至2010年10月VATS治疗SFFP患者19例,其中全胸腔镜手术14例,胸腔镜辅助小切口（6～8cm）手术3例,胸腔镜联合后外侧大切口（15cm）手术2例。结果全组均完整切除肿瘤,手术顺利,无并发症及围手术期死亡。术中证实13例肿瘤起源于脏层胸膜有蒂,5例起源于壁层胸膜无蒂宽基底,1例起源于纵隔胸膜有蒂。主要诊断依据免疫组化分析,19例患者的Vimentin和CD34均阳性表达;病理诊断良性17例,恶性2例。平均住院时间6d（4-11d）。术后平均随访51个月,无复发和转移。结论VATS创伤小,可作为治疗SFTP的首选方法,值得推荐。     

Diagnosis, treatment and long-term outcome of solitary fibrous tumours of the pleura.

OBJECTIVE: Solitary fibrous tumours of the pleura (SFTP) are rare and can histologically be differentiated into benign and malignant forms. The aim of this study is to present new cases, and discuss up-to-date preoperative examinations, the role of video-assisted thoracic surgery and long-term outcome. METHODS: Between 1993 and 2006, 27 SFTPs were diagnosed (14 females, mean age+/-SD, 62.3+/-9.6 years) at our institution. Medical records were reviewed, and follow-up was obtained by repeated examinations or contact with general practitioners. RESULTS: SFTPs were associated with symptoms in 63% of all cases. In the six patients in which positron emission tomography (PET) with 18F-fluorodeoxyglucose (FDG) was performed preoperatively, malignant lesions were all found to be positive. Complete resection was achieved by video-assisted thoracic surgery in 15 and anterolateral thoracotomy in 12 patients. Mean hospital stay was shorter for patients operated by video-assisted thoracic surgery compared to thoracotomy, 4.5 (range 3-6) versus 7.5 (range 4-25) days, respectively (p<0.01). Histology revealed 17 benign and 10 malignant SFTP. Mean+/-SD tumour diameter of malignant SFTPs was larger than in benign forms, 11.9+/-7.1 versus 6.1+/-3.5 cm, respectively (p<0.01). Tumour recurrence was recognised in four patients with malignant SFTPs at a median time interval after surgery of 38 (range 6-122) months, two late deaths occurred resulting from tumour recurrences. CONCLUSIONS: SFTPs can be treated minimally invasively by video-assisted thoracic surgery with short hospital stay. Large SFTPs with increased FDG-uptake have a high likelihood for malignancy. Long-term follow-up is mandatory in malignant SFTPs because of late recurrences associated with death.     

Extrapleural pneumonectomy for relapsed solitary fibrous tumors of the pleura with pleural dissemination

We report a case of a 62-year-old female with a prior thoracotomy for solitary fibrous tumor of the diaphragmatic pleura. There was no clear evidence of malignant solitary fibrous tumor of the pleura (SFTP). In the 19th postoperative month, she had a disseminated recurrence of SFTP in the left thoracic cavity. There was no evidence of metastasis from medical imaging. Accordingly, a left extrapleural pneumonectomy was performed. Pathological examination revealed a disseminated recurrence of malignant SFTP, showing a higher grade of malignancy, because the resected specimen was identical to the only section suspicious of malignancy in the previous tumor. She had no complaint and kept better performance status until the 7th postoperative month after the re-resection, when she had a recurrence in the left thoracic cavity and dissemination in the peritoneal cavity. She died of the recurrence 15 months after the re-resection and 34 months after the prior thoracotomy.     

Solitäre fibröse Pleuratumoren

Solitary fibrous tumors of the pleura (SFTP) are very rare neoplasms. The majority of these tumors are benign, but about 10-20 % fulfill the criteria of malignancy. The clinical presentation varies according to the size and intrathoracic localization. In early stages, often asymptomatic, the tumors may grow to an enormous size and then cause symptoms such as cough, chest pain and dyspnea, but also paraneoplastic syndromes such as hypoglycemia or digital clubbing. Between 1981 and June 1998 we treated in our institution 16 SFTP in 14 patients (4 M, 10 F, average age at first operation 58 years). Eight patients showed symptoms, whereas in the other cases the tumors were found on routine chest X-rays. The usually pedunculated SFTP were completely resected without complications. Two patients developed malignant recurrences, which infiltrated the right upper lung lobe and the diaphragm respectively. In these cases the tumor was resected together with the adjacent structures. Since late recurrences are more often malignant than primary SFTPs long-term follow-up is mandatory even in benign lesions.     

An operated case of malignant localized mesothelioma of the pleura]

An operable case of pedunculated localized mesothelioma of the pleura, a 62-year-old male, came to our clinic with chief complaint of chest X-ray abnormal shadow. On suspicion of pleural tumor, resection was performed. The operative findings revealed that the tumor was arising from visceral pleura of S1 + 2 a segment of left upper lobe, and didn't invade into peripheral tissue. The microscopic findings revealed that the tumor was consist of spindle tumor cells and capillary-like lesions, and had high cellularity and many mitosis. The tumor was diagnosed as localized malignant mesothelioma. Immunohistochemical stainings were performed using six monoclonal antibodies, vimentin, CEA, EMA, keratin (AE1, AE3), Leu-M1. Only vimentin reacted with tumor cells.     

Giant Solitary Fibrous Tumor of the Pleura (SFTPs) associated with paraneoplastic Doege Potter Syndrome

SFTPs are uncommon spindle cell mesenchymal tumors. These tumors arise in pleural as well as in extra pleural sites like liver, orbit, lung, nasal passages, meninges, respiratory tract, thyroid, soft tissues, major salivary glands, kidney, periosteum and spinal cord. SFTPs of thorax are interesting in that they can present with mass effect and paraneoplastic syndrome. Doege Potter Syndrome that is Giant SFTP presenting as hypoglycemic coma is an exceptional presentation of SFTPs. SFTPs are often mistaken for epithelial mesotheliomas. Positivity for vimentin, a marker of mesenchymal cells, negativity for cytoplasmic keratin which is positive in mesotheliomas and expression of Clusters of Differentiation (CD) 34 antigens can confirm the presence of a SFTP. Ultra structural studies further support the fibroblastic nature of these tumors. We hereby report an exceptional case of a 50-year-old lady with giant SFTP presenting with hypoglycemic coma. A diagnostic as well as therapeutic thoracotomy with complete surgical resection of the tumor was performed with complete relief of symptoms.     

Immunohistochemistry and surgical approaches in solitary fibrous tumor of the pleura

Solitary fibrous tumor of the pleura (SFTP) is a rare, benign, slow-growing neoplasm that arises from the submesothelial cells of the pleura. Usually, resection of the tumor and adjacent structures are sufficient for resolution. Nowadays, videothoracoscopy (VTC) allows adequate access for the surgical treatment of these tumors. CD34 antigen positivity is a differential feature with mesothelioma. We present our experience with 15 patients with SFTP (nine women and six men) who underwent surgical resection in the last 12 years (10 thoracotomies, one sternotomy and four VTC). Only four patients were symptomatic at diagnosis. In our opinion, VTC is a less invasive diagnostic and therapeutic approach than thoracotomy that provides an adequate approach for the resection of SFTP in selected patients. Because of the malignant potential of this tumor, long-term follow-up is mandatory.     

Solitary fibrous tumor of the pleura: morphogenesis and progression. A report of 36 cases

Purpose

We attempted to identify the exact point of tumor eruption of a solitary fibrous tumor of the pleura (SFTP).

Methods

We morphologically classified 36 SFTPs into 5 categories. Type A showed a connection that included a bloodstream with the pleura on both sides. Type B only showed a connection that included a bloodstream with the visceral pleura, and had a non-bloodstream connection with the parietal pleura. Type C only showed a connection that included a bloodstream with the visceral pleura, and had no connection with the parietal pleura. Type D showed a non-bloodstream connection with the visceral pleura, and only showed a connection that included a bloodstream with the parietal pleura. Finally, type E had no connection with the visceral pleura, and only showed a connection that included a bloodstream with the parietal pleura. The clinicopathological profiles of the tumors were investigated according to their type.

Results

The distribution of the 36 SFTPs was as follows: A (19 %), B (6 %), C (67 %), D (0 %) and E (8 %). The tumors categorized as type A tended to be large in size.

Conclusions

SFTPs commonly arise from the visceral pleura and in accordance with tumor progression they will form a non-bloodstream connection with the parietal pleura. Finally, a vascular pedicle will arise with the parietal pleura.

     

Giant solitary fibrous tumor of the pleura

Primitive neoplasms of the pleura are uncommon. Solitary fibrous tumor of the pleura (SFTP) is a benign variety of primitive pleural tumor, which is usually asymptomatic and discovered as an incidental finding. However, these tumors can sometimes become locally aggressive and can even be life-threatening. The treatment of choice is surgical resection. We report a case of SFTP in a 78-year-old woman with no relevant history, who presented with marked respiratory insufficiency and who required extended posterolateral thoracotomy to achieve complete resection of an SFTP weighing 2 kg.     

Giant Solitary Fibrous Tumor of the Pleura: An Analysis of Five Patients

Background  

Solitary fibrous tumor of the pleura (SFTP) represents a clinical entity rarely encountered, especially in giant forms. Complete surgical resection for giant tumor of pleura is a challenge. The aim of this article is to present five new cases of giant SFTP, and to discuss their clinical characteristics and the treatment strategy of such neoplasms.     

胸膜孤立性纤维瘤的诊治

目的 探讨胸膜孤立性纤维瘤的诊断和治疗方法.方法 回顾性分析2002年至2007年10例胸膜孤立性纤维瘤病人的临床和病理资料.10例中男3例,女7例.术前行超声引导下粗针穿刺活检明确诊断2例.全组均行手术治疗,其中3例行胸腔镜手术切除.结果 组织病理学报告,良性和恶性肿瘤各5例;恶性肿瘤CD34表达阳性率较低(3/5例),其中CD34阴性者nestin表达均阳性.失访1例,其余9例随访6～35个月,平均17.3个月,复发1例,死于脑转移1例.结论 超声引导下粗针穿刺结合免疫组化检查是术前明确诊断的一种较好方法.对于较小带蒂的肿瘤,胸腔镜手术是最佳手术方法.CD34阴性,同时nestin表达阳性可能是胸膜孤立性纤维瘤的一个恶性指标.     

Intrathoracic giant solitary fibrous tumor of the pleura: Case report

IntroductionSolitary Fibrous Tumor of the Pleura(SFTP) is exceedingly rare mesenchymal tumor commonly arising from the visceral pleura and accounts for <5% of all pleural tumors. Although it commonly has benign histologic characteristics, the tumor behavior is often unpredictable and less understood due to limited number of cases.Case presentationWe present a rare case of Intrathoracic Giant SFTP in a 65 years old female who presented with a progressive worsening of shortness of breath of 1 year duration associated with intermittent dry cough, low grade fever, easy fatigability and loss of appetite. Complete enbloc resection was done and she was discharged improved.DiscussionMost patients with SFTP are asymptomatic and definitive diagnosis is often made after surgical exploration and histopathologic study. Although 80% of SFTP arise from visceral pleura, the origin in our case was from the parietal pleura which is rare.ConclusionSFTP should be considered as differential diagnosis in patients with atypical or recurrent respiratory symptoms despite adequate medical treatment. Complete surgical excision is the main stay of treatment and meticulous post-operative follow up is mandatory as the risk of recurrence is higher and the tumor behavior is still less understood.     

Non-Hodgkin's lymphoma arising from the wall of old tuberculous empyema--a surgical case report

A 71-year-old male underwent therapeutic pneumothorax for left pulmonary tuberculosis 42 years ago. He visited our hospital in February 1988 with a complaint of hemosputum. In October, cytology of sputum revealed malignant cells, and the patient was admitted to our hospital for further examination. Because malignant cells were found by the left bronchial lavage, pan-pleuropneumonectomy was performed on January 12, 1989 under the diagnosis of left lung cancer. The tumor was partially left unremoved. Histological diagnosis was diffuse large cell type, B cell non-Hodgkin's lymphoma. Postoperatively, 2 courses of cyclophosphamide, adriamycin, vincristine, prednisone, etoposide (CHOP and VP-16) therapy were performed. However, the patient died of respiratory insufficiency on the 125th postoperative day. Recently, cases of malignant lymphoma involving the pleura after the old tuberculous empyema and therapeutic pneumothorax have been increased. Therefore, prompt diagnosis and treatment are recommended when tumor shadow is suspected as a result of imaging examination.     

Solitary fibrous tumor of the thymus with local invasiveness and pleural dissemination: report of a case.

A 74-year-old asymptomatic female presented with an anterior mediastinal mass incidentally discovered on a routine chest X-ray. Systemic evaluation demonstrated no metastatic lesions. The patient underwent an extended thymectomy via median sternotomy on suspicion of a thymoma. The tumor had arisen from the left half of the thymus without a pedicle and had directly invaded into the left lung and pericardium. The tumor was resected with the entire thymic tissue, and the invaded lung and pericardium were resected en-bloc. The size of the tumor was 5.3x4.0 cm. A disseminated lesion on the mediastinal pleura was also resected. Histopathologically, the lesion mainly consisted of non-atypical spindle-shaped tumor cells in a so-called "patternless pattern" with various densities of collagenous background. Pleomorphism and mitoses were not significant. Immunohistochemical analysis revealed mesenchymal positive markers such as vimentin and CD34. Epithelial markers such as CAM 5.2 and AE1/AE3 were negative. S-100 protein and desmin were not stained. Solitary fibrous tumor of the thymus was diagnosed histologically. Postoperative adjuvant chemotherapy or radiotherapy was not undertaken because the benefits were uncertain. She is well without recurrence 3 months after the operation.     

A case of primary leiomyosarcoma of the chest wall successfully resected under the video-assisted thoracoscopic approach.

We report a case of a 62-year-old woman with primary leiomyosarcoma of the chest wall which was successfully resected under the video-assisted thoracoscopic approach. The disease was found during the treatment for a malignant melanoma of the left heel. On the preoperative CT images, the lesion was suspected to be a metastasis of the malignant melanoma. The thoracoscopic surgery revealed that the tumor originated from the parietal pleura, and it was resected with a 5-mm margin of normal pleura. Histopathologically, the tumor was diagnosed as low-grade leiomyosarcoma. Since no residual tumor cells were proven in the resected margins histologically, further resection was not performed. At present, she is alive and well with no sign of recurrence of leiomyosarcoma two years and one month after operation. Thoracoscopic surgery is worth trying for accurate diagnosis of and effective treatment for a chest lesion without apparent invasion of the chest wall on the preoperative CT images.     

A case of malignant aortico-pulmonary paraganglioma

A 64-year-old male was admitted to our hospital suffering from dysphagia and dyspnea. The chest X-ray films and chest CT films revealed huge tumor in his upper-anterior mediastinum. The filling defect at lower rim of the left brachiocephalic vein on venography indicated malignant potential of the tumor. There was no abnormal value in blood and urine examinations except CA 19-9 which was 31.8 U/ml. The tumor was resected with bilateral pleura, pericardium and left brachiocephalic vein. Macroscopically the specimen accompanied with a lot of bleeding spots was soft and yellow and has no capsule. The size of tumor was 15 X 13 X 8 cm and its weight was 865 gm. No tumor recurrence was found until present time: two years after surgical therapy followed by adjuvant radiation therapy (60 Gray). Though histological findings of anterior part of the tumor were consisted of "Zellballen" cells and bleedings, the posterior part had cell atypia and invasion into vessels. These cells were slightly positive on Keratin stain and on NSE stain. Typical findings of paraganglioma were also indicated by electron microscopy: those findings were more chief cells, fewer sustentacular cells, abundant mitochondria and few dense-cored granules which are thought to secrete hormonal substances. Judging from these clinical and pathological results, this tumor was diagnosed nonfunctional aortico-pulmonary paraganglioma. Mediastinal paraganglioma is a very rare tumor. There is no report of malignant aortico-pulmonary paraganglioma in Japan after Glenner and Grimelys' study concerning extra-adrenal paraganglion system.     

Solitary fibrous tumor of the pleura with associated Doege–Potter syndrome

Doege–Potter syndrome is a paraneoplastic syndrome characterized by tumor-associated hypoglycemia secondary to a solitary fibrous tumor of the pleura. We present a case of an 84-year-old man, who presented with acute mental confusion and therapy-resistant hypoglycemia. Diagnostic imaging revealed a large sharply defined pleural tumor based on the left diaphragm, after surgical resection the diagnosis was made of a malignant solitary fibrous tumor of the pleura and restoration of the glucose homeostasis was observed.